Chlorinated paraffins (CPs), a class of persistent, toxic, and bioaccumulated compounds, have received increasing attention for their environmental occurrence and ecological and human health risks worldwide in the past decades. Understanding the environmental behavior and fate of CPs faces a huge challenge owing to the extremely complex CP congeners. Consequently, the aims of the present study are to summarize and integrate the bioaccumulation and biotransformation of CPs, including the occurrence of CPs in biota, tissue distribution, biomagnification, and trophic transfer, and biotransformation of CPs in plants, invertebrates, and vertebrates in detail. Biota samples collected in China showed higher CP concentrations than other regions, which is consistent with their huge production and usage. The lipid content is the major factor that determines the physical burden of CPs in tissues or organs. Regarding the bioaccumulation of CPs and their influence factors, inconsistent results were obtained. Biotransformation is an important reason for this variable. Some CP congeners are readily biodegradable in plants, animals, and microorganisms. Hydroxylation, dechlorination, chlorine rearrangement, and carbon chain decomposition are potential biotransformation pathways for the CP congeners. Knowledge of the influence of chain length, chlorination degree, constitution, and stereochemistry on the tissue distribution, bioaccumulation, and biotransformation is still scarce.